Definition of psoriasis severity in routine clinical care: current guidelines fail to capture the complexity of long-term psoriasis management.

BACKGROUND: Assessment of disease severity is an essential component of psoriasis management. Moderate-to-severe disease qualifies for systemic treatment but different definitions are used. OBJECTIVES: To analyse the impact of different severity definitions for psoriasis in real-world healthcare. METHODS: Cross-sectional data on 3274 patients with psoriasis from more than 200 dermatology offices and clinics across Germany were analysed for disease severity based on Psoriasis Area and Severity Index (PASI) and Dermatology Life Quality Index (DLQI). The proportions of patients having moderate-to-severe disease were determined accordingly. RESULTS: The proportion of patients meeting the European consensus criteria for moderate-to-severe psoriasis (PASI AND DLQI > 10) was 14·0%, although 45·3% attained at least PASI OR DLQI > 10. Consideration of all patients on systemic drugs as being 'moderate-to-severe' increased these proportions to 56·9% and 75·2%, respectively. When only PASI > 10 was used, moderate-to-severe disease affected 35·3% and 69·3%, respectively. CONCLUSIONS: The proportion of patients with psoriasis under dermatological care considered to have moderate-to-severe disease varies considerably according to how the latter is defined, resulting in uncertainty and inequity of access to systemic therapy. We propose an international standardisation in this for the sake of more reliable treatment and healthcare planning.

The German National Program on Psoriasis Health Care 2005-2015: results and experiences.

In 2005, the first national psoriasis survey in Germany revealed large deficits in health care particularly in patients with moderate to severe disease. The consecutive goal was to improve health care for psoriasis countrywide. For this, a large-scale national program was initiated starting with a comprehensive analysis of structures and processes of care for psoriasis. Patient burden, economic impact and barriers to care were systematically analyzed. In order to optimize routine care, a S3 guideline, a set of outcomes measures and treatment goals, were developed. Implementation was enforced by the German Psoriasis Networks (PsoNet) connecting the most dedicated dermatologists. The annual National Conference on Health Care in Psoriasis established in 2009 consented National Health Care Goals in Psoriasis 2010-2015 and defined a set of quality indicators, which are monitored on a regular basis. Currently 28 regional networks including more than 800 dermatologists are active. Between 2005 and 2014 7 out of 8 quality indicators have markedly improved, and regional disparities were resolved. e.g., mean PASI (Psoriasis Area Severity Index) dropped from 11.4 to 8.1 and DLQI (Dermatology Life Quality Index) from 8.6 to 5.9. A decade of experience indicates that a coordinated nationwide psoriasis program based on goal orientation can contribute to better quality of care and optimized outcomes.

Orcein and litmus.

Orcein was separated into 14 dyes by partition chromatography. Their constitutions were determined mainly by spectroscopy and led to formulae that are derived from 7-amino-2-phenoxazone, 7-hydroxy-2-phenoxazone, and 7-amino-2-phenoxazime, and that were confirmed by syntheses. The major constituent of litmus is assembled polymerically from 7-hydroxy-2-phenoxazone chromophores. The mechanism of formation is elucidated.

Disulfide bond mutations in follicle-stimulating hormone result in uncoupling of biological activity from intracellular behavior.

The crystal structure of human CG reveals that each subunit is a member of the superfamily of cystine-knot growth factors. Although the distribution of the cysteine residues in all the beta-subunits is conserved, the conformation of the human FSH dimer differs from that of the CG/LH dimers. This suggests that the function of the cystine bonded loops in the human FSHbeta-subunit may differ from that in the CGbeta-subunit. To address this issue, we deleted two disulfide bonds in the FSHbeta domain: cys 20-104 and cys 28-82, which correspond to the disulfide bonds 26-110 and 34-88, respectively, in the CGbeta-subunit. The cys 26-110 bond is associated with the "seat-belt" region and cys 34-88 is a bond in the cystine knot. Coexpression of the wild-type alpha-subunit with the FSHbeta cysteine mutants in CHO cells revealed no detectable heterodimer. The FSHbeta mutants were then incorporated into a single chain where the beta-subunit is genetically fused to the alpha-subunit. In such a model, the rate-limiting subunit assembly step is by-passed and mutations that otherwise block heterodimer formation can be evaluated in terms of biological activity. Compared with the nonmutated single chain, the single-chain 28-82 mutant is secreted more slowly and its recovery is substantially reduced, whereas secretion and recovery of the 20-104 mutant was not significantly affected. The receptor binding affinity of the cys 28-82 mutant did not differ from wild-type and binding of the cys 20-104 mutant was decreased only 2-fold. The signal transduction data parallel the binding affinities, although the maximal accumulation of cAMP is less for the cys 20-104 mutant than that seen for cys 28-82 and nonmutated single-chains variants. These data support the hypothesis that the determinants for intracellular behavior and bioactivity of the gonadotropins are not the same, and that the cystine knot is a critical determinant for the formation of a stable, assembly-competent subunit. In addition, the data imply that the "seat-belt" conformation does not play a prominent role in the bioactivity of FSH.

[Diagnostic and prognostic contribution of somatosensory evoked potentials by truncular and dermatomal stimulation in lumbosacral radiculopathy. Apropos of 120 cases surgically-treated]. / L'apport diagnostique et pronostique des potentiels évoqués somesthésiques par stimulation tronculaire et dermatomale dans les conflits disco-radiculaires lombaires. A propos de 120 cas opérés.

Lower-limb somatosensory evoked potentials (SEPs) of nerve-trunk (peroneal nerve, posterior tibial nerve) and dermatomal (L5 and S1) stimulation were recorded in 120 patients suffering from lumbosacral disk disease. Recordings were performed before surgery and on Days 4 and 50 after surgery. Electromyography was also performed before surgery. Dermatomal SEPs were shown to be sensitive (70%) and specific in both terms of their lateralizing value and their ability to identify the L5 or S1 level. Moreover, combining SEPs and EMG significantly increased the sensitivity of the electrophysiological testing when compared with each method alone. There was no relationship between SEPs performed before surgery and surgical outcome. Inversely, we demonstrated the prognostic value of SEPs performed on Day 4, SEP normalization at Day 4 was associated with a good outcome at Day 50 in 91.8% of examined cases, while the persistence of pathological SEPs at Day 4 was associated with poor outcome at Day 50 in 56% of the cases.

[Proteus syndrome]. / Proteus-Syndrom.

Proteus syndrome is a rare congenital disorder in which the major clinical manifestations are skeletal deformities and hamartomatous tumors. The case of a 42-year-old woman with right hemihypertrophy, macrodactyly of the right hallux, extreme kyphoscoliosis and bilateral plantar connective tissue naevi is reported. Proteus syndrome is reviewed with reference to this patient.